Bottom Line:
We report the imaging findings in a case of fetal lymphangioma involving the retroperitoneum and right lower extremity, and diagnosed by ultrasonography and magnetic resonance (MR) imaging at 26 weeks of gestation.Prenatal ultrasonograms and T2-weighted single-shot fast spin-echo MR images clearly revealed an extensive, multilocular cystic mass with internal hemorrhage in the retroperitoneum extending to the lower extremity.

Affiliation: Department of Radiology, The Catholic University of Korea, College of Medicine, Seoul, Korea. jybyun@catholic.ac.kr

ABSTRACTWe report the imaging findings in a case of fetal lymphangioma involving the retroperitoneum and right lower extremity, and diagnosed by ultrasonography and magnetic resonance (MR) imaging at 26 weeks of gestation. Prenatal ultrasonograms and T2-weighted single-shot fast spin-echo MR images clearly revealed an extensive, multilocular cystic mass with internal hemorrhage in the retroperitoneum extending to the lower extremity.

Figure 1: A 26-week-old female fetus with an extensive lymphangioma.A. Prenatal ultrasonography shows a large multiseptated cystic mass (arrows) with variable echo patterns, located at the right of the fetal abdomen and also involving the right lower extremity (open arrows). The transverse diameter of this extremity is much greater than that of the left one (L). Color Doppler ultrasonography revealed no flow within the mass at the time of examination (not shown).B. Sagittal T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) depicts a large intra-abdominal high-signal-intensity mass (arrows) with multiple internal locules, displacing the ipsilateral kidney (K) superiorly. The mass (arrows) extends continuously to the right buttock and right lower extremity.C. Coronal T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) clearly reveals the multilocular cystic nature of the mass (arrows), each locule of which contains multiple fluid-fluid levels. S=Stomach.D. T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) obtained parallel to the right lower fetal extremity shows the distal extent of the lymphangioma (arrows). This presents as an extensive subcutaneous cystic mass, with asymmetric limb hypertrophy.E. Post-mortem photograph depicts a large lymphangioma (arrows) at the right of the abdomen, extending to the right lower extremity.

Mentions:
A 34-year-old woman whose one previous pregnancy had involved cesarian section and the delivery of a healthy male infant at term was referred to our hospital at 26 weeks of gestation on account of earlier sonographic findings which had indicated possible multicystic dysplastic kidney and sacrococcygeal teratoma. Detailed sonography revealed the presence of a large, multiseptated cystic mass at the right side of the abdomen, extending to the right buttock and lower extremity, the transverse diameter of which was much larger than that of the left (Fig. 1A). The mass demonstrated variable echo patterns; color Doppler sonography revealed no internal flow at the time of examination. For further evaluation of these complex abnormalities, MR imaging was performed using a 1.5-T superconducting unit (Signa Infinity with twin-speed dual gradient; General Electric Medical Systems, Milwaukee, Wis., U.S.A.) together with a phased array coil. After performing a localized gradient-echo sequence, T2-weighted single-shot fast spin-echo MR images (TR/TE, 1624/79; bandwidth, 31.2 kHz; field of view, 22×22 cm; matrix, 224×192; slice thickness, 5 mm; intersection gap, 0 mm; number of excitations, 0.56) were obtained in the axial, coronal, and sagittal planes; the acquisition time for each was less than 1 second. The images revealed a large, multilocular, high-signal mass in the right-side of the abdomen; displacement of the ipsilateral kidney superiorly and bowel loops to the left was noted (Figs. 1B, C). Within the mass, which extended continuously to the right buttock and right lower extremity, presenting as an extensive subcutaneous cystic mass and causing asymmetric limb hypertrophy, multiple fluid-fluid levels were noted (Figs. 1C, D). Cystic lymphangioma was indicated, and after counselling, the parents opted for termination of the pregnancy because of the poor prognosis predicted on the basis of the lesion's large size and infiltrative nature.

Figure 1: A 26-week-old female fetus with an extensive lymphangioma.A. Prenatal ultrasonography shows a large multiseptated cystic mass (arrows) with variable echo patterns, located at the right of the fetal abdomen and also involving the right lower extremity (open arrows). The transverse diameter of this extremity is much greater than that of the left one (L). Color Doppler ultrasonography revealed no flow within the mass at the time of examination (not shown).B. Sagittal T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) depicts a large intra-abdominal high-signal-intensity mass (arrows) with multiple internal locules, displacing the ipsilateral kidney (K) superiorly. The mass (arrows) extends continuously to the right buttock and right lower extremity.C. Coronal T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) clearly reveals the multilocular cystic nature of the mass (arrows), each locule of which contains multiple fluid-fluid levels. S=Stomach.D. T2-weighted single-shot fast spin-echo MR image (TR/TE, 1624/79) obtained parallel to the right lower fetal extremity shows the distal extent of the lymphangioma (arrows). This presents as an extensive subcutaneous cystic mass, with asymmetric limb hypertrophy.E. Post-mortem photograph depicts a large lymphangioma (arrows) at the right of the abdomen, extending to the right lower extremity.

Mentions:
A 34-year-old woman whose one previous pregnancy had involved cesarian section and the delivery of a healthy male infant at term was referred to our hospital at 26 weeks of gestation on account of earlier sonographic findings which had indicated possible multicystic dysplastic kidney and sacrococcygeal teratoma. Detailed sonography revealed the presence of a large, multiseptated cystic mass at the right side of the abdomen, extending to the right buttock and lower extremity, the transverse diameter of which was much larger than that of the left (Fig. 1A). The mass demonstrated variable echo patterns; color Doppler sonography revealed no internal flow at the time of examination. For further evaluation of these complex abnormalities, MR imaging was performed using a 1.5-T superconducting unit (Signa Infinity with twin-speed dual gradient; General Electric Medical Systems, Milwaukee, Wis., U.S.A.) together with a phased array coil. After performing a localized gradient-echo sequence, T2-weighted single-shot fast spin-echo MR images (TR/TE, 1624/79; bandwidth, 31.2 kHz; field of view, 22×22 cm; matrix, 224×192; slice thickness, 5 mm; intersection gap, 0 mm; number of excitations, 0.56) were obtained in the axial, coronal, and sagittal planes; the acquisition time for each was less than 1 second. The images revealed a large, multilocular, high-signal mass in the right-side of the abdomen; displacement of the ipsilateral kidney superiorly and bowel loops to the left was noted (Figs. 1B, C). Within the mass, which extended continuously to the right buttock and right lower extremity, presenting as an extensive subcutaneous cystic mass and causing asymmetric limb hypertrophy, multiple fluid-fluid levels were noted (Figs. 1C, D). Cystic lymphangioma was indicated, and after counselling, the parents opted for termination of the pregnancy because of the poor prognosis predicted on the basis of the lesion's large size and infiltrative nature.

Bottom Line:
We report the imaging findings in a case of fetal lymphangioma involving the retroperitoneum and right lower extremity, and diagnosed by ultrasonography and magnetic resonance (MR) imaging at 26 weeks of gestation.Prenatal ultrasonograms and T2-weighted single-shot fast spin-echo MR images clearly revealed an extensive, multilocular cystic mass with internal hemorrhage in the retroperitoneum extending to the lower extremity.

Affiliation:
Department of Radiology, The Catholic University of Korea, College of Medicine, Seoul, Korea. jybyun@catholic.ac.kr

ABSTRACTWe report the imaging findings in a case of fetal lymphangioma involving the retroperitoneum and right lower extremity, and diagnosed by ultrasonography and magnetic resonance (MR) imaging at 26 weeks of gestation. Prenatal ultrasonograms and T2-weighted single-shot fast spin-echo MR images clearly revealed an extensive, multilocular cystic mass with internal hemorrhage in the retroperitoneum extending to the lower extremity.